Which is not a feature of paroxysmal nocturnal hemoglobinuria ?
**Core Concept**
Paroxysmal nocturnal hemoglobinuria (PNH) is a rare acquired disorder characterized by the destruction of red blood cells, bone marrow failure, and the presence of complement-mediated hemolysis. It is caused by mutations in the PIGA gene, leading to the absence of glycosylphosphatidylinositol (GPI) anchors on the surface of blood cells.
**Why the Correct Answer is Right**
PNH is characterized by three main features:
- **Hemolytic anemia**: The destruction of red blood cells, often triggered by the activation of the complement system on the surface of red blood cells.
- **Bone marrow failure**: PNH is associated with a range of bone marrow abnormalities, including aplastic anemia, which can lead to a decrease in blood cell production.
- **Thrombosis**: PNH patients are at an increased risk of developing thrombotic events, such as deep vein thrombosis and pulmonary embolism, due to the activation of endothelial cells and platelets.
**Why Each Wrong Option is Incorrect**
**Option A:** Hemolytic uremic syndrome (HUS) is not a feature of PNH, although both conditions involve complement-mediated damage to blood cells. HUS typically presents with thrombocytopenia, microangiopathic hemolytic anemia, and acute kidney injury, which are not characteristic of PNH.
**Option B:** Bone marrow failure is a feature of PNH, as mentioned earlier. PNH patients often present with aplastic anemia or other bone marrow abnormalities.
**Option C:** Thrombosis is indeed a feature of PNH, as it is associated with an increased risk of developing thrombotic events.
**Option D:** (no option D provided)
**Clinical Pearl / High-Yield Fact**
PNH is often associated with a deficiency of CD55 and CD59 on the surface of red blood cells, which are complement regulatory proteins that protect the cells from complement-mediated damage.
**Correct Answer:** A.